Biomedical subjects
Publications and source records attributed to H Sabio.
A 3-month-old female with bilateral adrenal neuroblastoma had a unilateral adrenalectomy followed by cyclophosphamide treatment for 2 years. At 7, she has no evidence of disease. The favorable course of this patient and that of another young child reported with bilateral neuroblastoma as well as wide-spread metastases suggests that bilateral adrenalectomy may not be necessary for a child under 1 year of age with bilateral disease.
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Two brothers with the X-linked disorder, dyskeratosis congenita, are described. They showed the dermatologic triad of reticular hyperpigmentation, dystrophic nails, and leukoplakia oris as well as the other major feature of this disorder, aplastic anemia. Less common features observed included prenatal and postnatal growth retardation, mental retardation, elevated immunoglobulin levels, and gastrointestinal hemorrhage from mucosal ulceration. Previously unreported findings were intracranial calcifications and nutmeg-like cirrhotic changes of the liver. These brothers demonstrated that skeletal changes and bony fragility may predate anemia or steroid therapy. Although a DNA repair defect is postulated as a possible primary defect, cytogenetic studies revealed no evidence of increased chromosomal breakage.
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The successful induction and maintenance of hematologic remission in childhood acute leukemia has led to the recognition of extramedullary leukemia as a major clinical problem [1-6]. The central nervous system is the most common site of extramedullary relapse [1,7-12]. A more recently recognized form of extramedullary relapse is the testis [13-22]. This paper will review a series of patients with childhood acute leukemia who suffered testicular relapse. Modes of therapy for testicular relapse and research conducted into the etiology of testicular relapse will be discussed.
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A 27 month old white male infant clinically suspected of having histiocytosis X had an enlarged hard hypofunctioning thyroid. A needle biopsy of the thyroid showed disruption of the thyroid architecture by an infiltrate consisting of large histiocytes admixed with eosinophils, neutrophils, and lymphocytes, confirming the clinical diagnosis of histiocytosis X.
A case of Wilms' tumor presenting with direct intracardiac extension is described. The clinical features of this unusual complication are described from a review of previous cases. The presence of cardiac abnormalities in a child with a right-sided Wilms' tumor can suggest direct intracardiac extension.
Spontaneous platelet aggregation (SPA), a phenomenon observed in vitro, was noted in 29 of 74 (39%) children and adolescents with insulin-dependent diabetes. Its occurrence was independent of age of onset, duration of diabetes, HbAI levels or presence of background retinopathy but varied between normal and abnormal in given individuals tested repeatedly over some time. Plasma beta-thromboglobulin, the platelet protein specific for Phase II of the release reaction, was elevated in the diabetic subjects but was independent of the occurrence of SPA. SPA in the diabetic children and adolescents, in contrast to SPA in other conditions, was not suppressed by either acute or chronic administration of aspirin adequate to suppress ADP induced second wave aggregation. SPA was uniformly suppressed by addition to the in vitro system of EDTA, adenosine, antisera to human fibrinogen or vitamin E. The data suggest that SPA is ADP induced and reflects either abnormal platelet membrane permeability to the nucleotide or its abnormal release, or abnormal platelet sensitivity to normal plasma levels of ADP.
Children with acute promyelocytic leukemia were treated with aggressive daunomycin induction and 6-mercaptopurine and methotrexate maintenance. The remission rate was 8 out of 10 fully evaluable patients. Three are still alive up to 32 months. With vigorous induction therapy, occurrence of disseminated intravascular coagulation was rare. Only one case of DIC was seen in a child who had pseudomonas sepsis at time of diagnosis. Since current protocols are more aggressive, future chemotherapy include patients with acute promyelocytic leukemia.
The response of nucleated bone marrow cells from C3H mice to hyperthermic temperatures of 41.5 to 49.5 degrees for a fixed heating period of 30 min has been determined. The threshold temperatures for cell lysis, loss of trypan blue exclusion, and histological evidence of cell injury were greater than 49.5 degrees, 45.5 degrees and 43.5 degrees, respectively. Growth of mature granulocyte-monocytes from precursors was evaluated in Millipore diffusion chamber culture. There was a biphasic decrease in granulocyte-monocyte growth after exposure to temperatures of 41.5 to 45.5 degrees. The ratio of granulocytes to monocytes in proliferated cultures was not altered after hyperthermia. Pluripotent and committed granuloid stem cells were assayed by the spleen colony and plasma clot diffusion chamber techniques. These also showed a biphasic decrease with increase in temperature, with committed stem cells having a greater thermal sensitivity at lower temperatures.
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Growth hormone (G.H.) or a G.H.-dependent somatomedin may be involved in the process of acute lymphoblastic leukaemia (A.L.L.). Growth hormone has a trophic effect on lymphoid tissue and also specific receptors on lymphocytes, most probably T cells. Hypophycess. Resting concentrations of G.H. and somatomedin activity are raised in some children with A.L.L. and may be reduced after remission is achieved. It is suggested that control of G.H. and/or somatomedin concentrations may be necessary for adequate treatment of some cases of A.L.L. in children.
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